0000000000625979

AUTHOR

Christian Lampe

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Development of a Scoring System to Evaluate the Severity of Craniocervical Spinal Cord Compression in Patients with Mucopolysaccharidosis IVA (Morqui…

2012

BackgroundAs spinal cord compression at the craniocervical junction (CCJ) is a life-threatening manifestation in patients with mucopolysaccharidosis (MPS) IVA, surgical decompression should be performed before damage becomes irreversible. We evaluated the diagnostic value of several examinations for determining the need for decompression surgery.MethodsWe retrospectively analysed results of clinical neurological examination, somatosensory evoked potential (SEP) and magnetic resonance imaging (MRI) in 28 MPS IVA patients. A scoring system - based on the severity of findings - was used to compare results of patients with and without indication for decompression surgery. Individual test scores…

musculoskeletal diseasesmedicine.medical_specialtyScoring systembusiness.industryMucopolysaccharidosisNeurosciencesMorquio A syndromeCraniocervical junctionNeurodegenerativemedicine.diseaseMedian nerveArticleSurgerySpinal cord compressionDecompressive surgerymedicineIn patientSpinal Cord InjurybusinessJIMD reports
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